Skeletal Radio1 (1992) 21:335-338
Skeletal Radiology
Case report 740 Elaine S. Gould, M.D. 1, James M. Cooper, M.D. 1, Hollis G. Potter, M.D. 1, Lewis B. Lane, M.D. 2, Wilhelmina Cruz-Vetrano, M.D. 3, Manjula Bansal, M.D. '~, and Alessandro Franchi, M.D. 4 Departments of 1 Radiology, 2 Orthopaedics, and 3 Pathology, North Shore University Hospital/Cornell University Medical College, Manhasset, New York, 4 Department of Pathology, Hospital for Special Surgery, New York, USA
Clinical information
Imaging studies Fig. 1. Frontal radiograph of the left wrist demonstrates a lytic lesion of the hamate bone
Fig. 2. Lateral radiographs of the left wrist show slight thinning and disruption of the dorsal cortex (arrow), with some expansion
This 80-year-old man presented with pain in the left wrist of approximately 6 months' duration prior to seeking medical advice. His past medical history was significant for emphysema, carcinoma of the bladder, gastrectomy and vagotomy. Physical examination revealed tenderness over the dorsum of the wrist with minimal swelling. L a b o r a t o r y data were unremarkable. Radiographs of the left wrist (Fig. 1) demonstrated a lucent lesion occupying the body of the hamate bone with thinning, slight expansion, and disruption of the dorsal cortex (Fig. 2, arrow). No associated mineralized matrix was present. The remainder of the carpus was unremarkable at initial presentation. The patient underwent biopsy of the lesion in the hamate.
Address reprint requests to: Elaine S. Gould,
M.D., Department of Radiology, North Shore University Hospital/Cornell University Medical College, 300 Community Drive, Manhasset, NY 11030, USA 9 1992 International Skeletal Society
336
Diagnosis: Giant cell tumor of the hamate bone The differential diagnosis included brown tumor of hyperparathyroidism, villonodular synovitis, intraosseous ganglion, giant cell reparative granuloma, pseudotumor of hemophilia, metastatic disease, posttraumatic cyst, gout, and, less likely, chondroid tumors, silicone synovitis, and tuberculosis. Operatively, on gross inspection, the dorsal cortex of the hamate was exceedingly thin, with a nondisplaced fracture. The tumor was red and friable, with involvement of marrow, cortex, and periosteum. The microscopic features of the tumor included a background of proliferating, homogeneous, mononuclear cells with multinucleated giant cells dispersed evenly throughout. The mononuclear component consisted of short-spindled, occasionally round cells, with ovoid to round nuclei, containing fine chromatin and rare nucleoli. The cytoplasm was faintly eosinophilic and foamy in appearance. Mitotic figures were rare. Islands of eroded bone as well as immature, poorly formed cartilage and osseous tissue were scattered in the tumor parenchyma (Fig. 3). The tumor displayed prominent vascularity in areas, and scattered hemosiderin pigments were noted. The tumor involved the bone extensively, including the submitted cortex with its surrounding periosteum. Excision of the lesion was performed with subsequent autogenous bone grafting obtained from the distal end of the radius. Initial workup included total body bone scan and computed tomography (CT) of the chest that demonstrated no evidence of metastatic disease. Approximately one and a half years later, the patient presented with increasing pain and swelling. Plain radiographs showed extensive destruction of the hamate, triquetrum, lunate, and the base of the fourth and fifth metacarpal bones (Fig. 4). The patient subsequently underwent a partial carpectomy. The specimen consisted of three carpal bones (hamate, lunate, and triquetrum) and proximal ends of the
E.S. Gould et al. : Case report 740
Pathological study
Fig. 3. High power photomicrograph demonstrates the homogeneous mononuclear stromal cells and evenly distributed, multinucleated giant cells ( x 400)
4th and 5th metacarpals, along with attached soft tissues measuring 6 x 6 x 4 cm. Multiple, red-brown, softtissue nodules of varying sizes adjacent to the bones were identified, the largest measuring 3.5 x 2.1 x 1.5 cm. On sectioning, bloody, brown, coagulated material was noted. A section through the bones revealed extensive destruction of both the cortical and cancellous components of the metacarpals, hamate, lunate and triquetrum (Fig. 5A). The corresponding radiograph of the specimen (Fig. 5B) confirmed these findings and further demonstrated the numerous cystic areas present within the bones as well as erosions at the periphery of the bones (Fig. 3), Microscopically, the histological features were similar to those observed initially (Fig. 4). The tumor was present within the subchondral bone with soft-tissue extension. Focal reactive bone formation was present. No atypical mitosis was present.
Discussion Giant cell tumor (GCT) of bone is not uncommon, accounting for approximately 5% of all primary bone
tumors and 21% of benign tumors [3]. Roughly, 75% of cases occur between the ages of 20 and 40 years. Giant cell tumor at the age of 80 years is extremely rare. The femaleto-male ratio is approximately 1.1 : 1 in the upper extremity [7]. Although the most common sites are around the knee, they are also not uncommonly seen in the sacrum, distal end of the radius, proximal end of the humerus, femur, and fibula [3]. Giant cell tumors of the hand and wrist, however, are uncommon, accounting for only 2% of all giant cell tumors. One series reported only 2 of 28 tumors occurring in the carpal bones. When they occur in there, they show a higher incidence of multicentricity, with a predilection for a younger age group, and tend to become symptomatic at an earlier stage [1]. In Averill etal.'s series of 28 GCTs of bones of the hand, the oldest person was 36 years old [1]. In Campanacci et al.'s [2] series of 327 patients with GCTs of bones of the hand and in McDonald et al.'s [6] series of 221 patients, no cases occurred after 80 years of age. FitzPatrick and Bullough described a GCT of the lunate bone in a 28-year-old woman [4].
337
E.S. Gould et al. : Case report 740
Further radiological and pathological studies
Fig. 4. Frontal radiograph of the left wrist performed approximately 1.5 years later, noting extensive lyric lesions in the hamate, lunate, triquetrum, and 4th and 5th metacarpal bases Fig. 5. A Cross-section of gross specimen. B Specimen radiograph shows extensive destruction involving 4th and 5th metacarpals, hamate (H), triquetrum (7) and lunate (L) Fig. 6. High power photomicrograph of specimen is similar to Fig. 3
Radiographically, GCTs are lucent, and when they occur in long bones, they are usually eccentric, with their epicenter in the epiphysis. They can, however, very rarely involve the metaphysis and even the diaphysis. No associated mineralized matrix is present. Commonly, bulging of the overlying cortex with a thin rim of subperiosteal new bone outlining the tumor is present. The margins may be either well- or ill-defined. A fine sclerotic border may be seen. No evi-
dence of periosteal reaction is noted unless an associated fracture occurs. The most c o m m o n presenting symptom of a G C T is pain. Some patients recall trivial trauma prior to the diagnosis being established. In the reported series by Averill et al. [1], the overall average duration of symptoms before radiographic or histological diagnosis ranged from 1 to 6 months in 58%, and 6 18 months in 42% of patients. In the hand, the duration of symptoms is
shorter, ranging from 4 days to 4 months, with a mean of 2 months [1]. Rates of recurrence vary with the type of treatment. In one series [2], the local recurrence rate was 27% after intralesional excision, 8% after marginal excision, and 0% when a radical procedure was formed. Pulmonary metastases occur in approximately 1 % - 2 % of patients, regardless of treatment. A grading system to correlate clinical outcome and
338 histological features was described by Jaffe et al. [5]. This was shown to be unreliable, and a relatively p o o r correlation of the recurrence rate with histological grading exists. According to Averill et al. [1], all G C T s of the hand should be considered locally aggressive, irrespective of the histological grades. This concept, however, is denied by some authorities on the subject. Histologically benign-appearing lesions can become locally aggressive or metastasize. O f the 15 patients who were treated by curettage, with or without bone grafting, 11% developed recurrence. O f the 7 patients who were treated with local resection, 3 suffered a recurrence. None o f the patients in the Averill et al. series had a recurrence following an amputation or a ray resection. In their series, 0.4% of all G C T s were multicentric. Approximately 39% of multicentric G C T s had at least one focus in the hand and 18% of G C T s of the hand had multicentric foci. Averill et al. con-
E.S. Gould et al. : Case report 740 cluded, therefore, that routine radionuclide bone scanning of the skeleton is recommended if a lesion is present within the hand. In the work-up of a patient with a G C T , plain radiography and either complex motion t o m o g r a p h y or CT scanning should be performed to evaluate the full extent of the lesion and its margins. Although the magnetic resonance (MR) characteristics of G C T s are nonspecific [3], this modality can add information as to the extent of the lesion both in bone and adjacent soft tissues. In summary, an unusual case of a G C T originating in the hamate bone in an 80-year-old m a n is presented. Following initial excision and autogenous bone grafting, subsequent extensive spread to adjacent carpal and metacarpal bones occurred. The differential diagnoses have been discussed. The rarity of this t u m o r in the carpus, the occurrence at the age of 80 years, the higher incidence of multicentricity, as
well as the recurrence rate following intralesional excision have been considered.
References 1. Averitl RM, Smith RJ, Campbell CJ (1980) Giant cell tumors of the bones of the hand. J Hand Surg 5:39 2. Campanacci M, Baldini N, Boriani S, Sudanese A (1987) Giant cell tumor of bone. J Bone Joint Surg [Am] 69:106 3. Carrasco CH, Murray JA (1989) Giant cell tumors. Orthop Clin North Am 20 (63) : 395 4. FitzPatrick DJ, Bullough PG (1977) Giant cell tumor of the lunate bone: a case report. J Hand Surg 2:269 5. Jaffe HL, Lichtenstein L, Portis RB (1940) Giant cell tumor of bone. Its pathologic appearance, grading, supposed ovarians and treatment. Arch Pathol 30 : 993 6. McDonald D J, Sim FH, McLeod RA, Dahlin DC (1986) Giant cell tumor of bone. J Bone Joint Surg [Am] 68:235 7. Moser RP, Kransdorf MJ, Gilkey FW, Manaster BJ (1990) From the archives of the AFIP giant cell tumor of the upper extremity. Radiographics 10:83
Skeletal Radiology
Case report 740 Elaine S. Gould, M.D. 1, James M. Cooper, M.D. 1, Hollis G. Potter, M.D. 1, Lewis B. Lane, M.D. 2, Wilhelmina Cruz-Vetrano, M.D. 3, Manjula Bansal, M.D. '~, and Alessandro Franchi, M.D. 4 Departments of 1 Radiology, 2 Orthopaedics, and 3 Pathology, North Shore University Hospital/Cornell University Medical College, Manhasset, New York, 4 Department of Pathology, Hospital for Special Surgery, New York, USA
Clinical information
Imaging studies Fig. 1. Frontal radiograph of the left wrist demonstrates a lytic lesion of the hamate bone
Fig. 2. Lateral radiographs of the left wrist show slight thinning and disruption of the dorsal cortex (arrow), with some expansion
This 80-year-old man presented with pain in the left wrist of approximately 6 months' duration prior to seeking medical advice. His past medical history was significant for emphysema, carcinoma of the bladder, gastrectomy and vagotomy. Physical examination revealed tenderness over the dorsum of the wrist with minimal swelling. L a b o r a t o r y data were unremarkable. Radiographs of the left wrist (Fig. 1) demonstrated a lucent lesion occupying the body of the hamate bone with thinning, slight expansion, and disruption of the dorsal cortex (Fig. 2, arrow). No associated mineralized matrix was present. The remainder of the carpus was unremarkable at initial presentation. The patient underwent biopsy of the lesion in the hamate.
Address reprint requests to: Elaine S. Gould,
M.D., Department of Radiology, North Shore University Hospital/Cornell University Medical College, 300 Community Drive, Manhasset, NY 11030, USA 9 1992 International Skeletal Society
336
Diagnosis: Giant cell tumor of the hamate bone The differential diagnosis included brown tumor of hyperparathyroidism, villonodular synovitis, intraosseous ganglion, giant cell reparative granuloma, pseudotumor of hemophilia, metastatic disease, posttraumatic cyst, gout, and, less likely, chondroid tumors, silicone synovitis, and tuberculosis. Operatively, on gross inspection, the dorsal cortex of the hamate was exceedingly thin, with a nondisplaced fracture. The tumor was red and friable, with involvement of marrow, cortex, and periosteum. The microscopic features of the tumor included a background of proliferating, homogeneous, mononuclear cells with multinucleated giant cells dispersed evenly throughout. The mononuclear component consisted of short-spindled, occasionally round cells, with ovoid to round nuclei, containing fine chromatin and rare nucleoli. The cytoplasm was faintly eosinophilic and foamy in appearance. Mitotic figures were rare. Islands of eroded bone as well as immature, poorly formed cartilage and osseous tissue were scattered in the tumor parenchyma (Fig. 3). The tumor displayed prominent vascularity in areas, and scattered hemosiderin pigments were noted. The tumor involved the bone extensively, including the submitted cortex with its surrounding periosteum. Excision of the lesion was performed with subsequent autogenous bone grafting obtained from the distal end of the radius. Initial workup included total body bone scan and computed tomography (CT) of the chest that demonstrated no evidence of metastatic disease. Approximately one and a half years later, the patient presented with increasing pain and swelling. Plain radiographs showed extensive destruction of the hamate, triquetrum, lunate, and the base of the fourth and fifth metacarpal bones (Fig. 4). The patient subsequently underwent a partial carpectomy. The specimen consisted of three carpal bones (hamate, lunate, and triquetrum) and proximal ends of the
E.S. Gould et al. : Case report 740
Pathological study
Fig. 3. High power photomicrograph demonstrates the homogeneous mononuclear stromal cells and evenly distributed, multinucleated giant cells ( x 400)
4th and 5th metacarpals, along with attached soft tissues measuring 6 x 6 x 4 cm. Multiple, red-brown, softtissue nodules of varying sizes adjacent to the bones were identified, the largest measuring 3.5 x 2.1 x 1.5 cm. On sectioning, bloody, brown, coagulated material was noted. A section through the bones revealed extensive destruction of both the cortical and cancellous components of the metacarpals, hamate, lunate and triquetrum (Fig. 5A). The corresponding radiograph of the specimen (Fig. 5B) confirmed these findings and further demonstrated the numerous cystic areas present within the bones as well as erosions at the periphery of the bones (Fig. 3), Microscopically, the histological features were similar to those observed initially (Fig. 4). The tumor was present within the subchondral bone with soft-tissue extension. Focal reactive bone formation was present. No atypical mitosis was present.
Discussion Giant cell tumor (GCT) of bone is not uncommon, accounting for approximately 5% of all primary bone
tumors and 21% of benign tumors [3]. Roughly, 75% of cases occur between the ages of 20 and 40 years. Giant cell tumor at the age of 80 years is extremely rare. The femaleto-male ratio is approximately 1.1 : 1 in the upper extremity [7]. Although the most common sites are around the knee, they are also not uncommonly seen in the sacrum, distal end of the radius, proximal end of the humerus, femur, and fibula [3]. Giant cell tumors of the hand and wrist, however, are uncommon, accounting for only 2% of all giant cell tumors. One series reported only 2 of 28 tumors occurring in the carpal bones. When they occur in there, they show a higher incidence of multicentricity, with a predilection for a younger age group, and tend to become symptomatic at an earlier stage [1]. In Averill etal.'s series of 28 GCTs of bones of the hand, the oldest person was 36 years old [1]. In Campanacci et al.'s [2] series of 327 patients with GCTs of bones of the hand and in McDonald et al.'s [6] series of 221 patients, no cases occurred after 80 years of age. FitzPatrick and Bullough described a GCT of the lunate bone in a 28-year-old woman [4].
337
E.S. Gould et al. : Case report 740
Further radiological and pathological studies
Fig. 4. Frontal radiograph of the left wrist performed approximately 1.5 years later, noting extensive lyric lesions in the hamate, lunate, triquetrum, and 4th and 5th metacarpal bases Fig. 5. A Cross-section of gross specimen. B Specimen radiograph shows extensive destruction involving 4th and 5th metacarpals, hamate (H), triquetrum (7) and lunate (L) Fig. 6. High power photomicrograph of specimen is similar to Fig. 3
Radiographically, GCTs are lucent, and when they occur in long bones, they are usually eccentric, with their epicenter in the epiphysis. They can, however, very rarely involve the metaphysis and even the diaphysis. No associated mineralized matrix is present. Commonly, bulging of the overlying cortex with a thin rim of subperiosteal new bone outlining the tumor is present. The margins may be either well- or ill-defined. A fine sclerotic border may be seen. No evi-
dence of periosteal reaction is noted unless an associated fracture occurs. The most c o m m o n presenting symptom of a G C T is pain. Some patients recall trivial trauma prior to the diagnosis being established. In the reported series by Averill et al. [1], the overall average duration of symptoms before radiographic or histological diagnosis ranged from 1 to 6 months in 58%, and 6 18 months in 42% of patients. In the hand, the duration of symptoms is
shorter, ranging from 4 days to 4 months, with a mean of 2 months [1]. Rates of recurrence vary with the type of treatment. In one series [2], the local recurrence rate was 27% after intralesional excision, 8% after marginal excision, and 0% when a radical procedure was formed. Pulmonary metastases occur in approximately 1 % - 2 % of patients, regardless of treatment. A grading system to correlate clinical outcome and
338 histological features was described by Jaffe et al. [5]. This was shown to be unreliable, and a relatively p o o r correlation of the recurrence rate with histological grading exists. According to Averill et al. [1], all G C T s of the hand should be considered locally aggressive, irrespective of the histological grades. This concept, however, is denied by some authorities on the subject. Histologically benign-appearing lesions can become locally aggressive or metastasize. O f the 15 patients who were treated by curettage, with or without bone grafting, 11% developed recurrence. O f the 7 patients who were treated with local resection, 3 suffered a recurrence. None o f the patients in the Averill et al. series had a recurrence following an amputation or a ray resection. In their series, 0.4% of all G C T s were multicentric. Approximately 39% of multicentric G C T s had at least one focus in the hand and 18% of G C T s of the hand had multicentric foci. Averill et al. con-
E.S. Gould et al. : Case report 740 cluded, therefore, that routine radionuclide bone scanning of the skeleton is recommended if a lesion is present within the hand. In the work-up of a patient with a G C T , plain radiography and either complex motion t o m o g r a p h y or CT scanning should be performed to evaluate the full extent of the lesion and its margins. Although the magnetic resonance (MR) characteristics of G C T s are nonspecific [3], this modality can add information as to the extent of the lesion both in bone and adjacent soft tissues. In summary, an unusual case of a G C T originating in the hamate bone in an 80-year-old m a n is presented. Following initial excision and autogenous bone grafting, subsequent extensive spread to adjacent carpal and metacarpal bones occurred. The differential diagnoses have been discussed. The rarity of this t u m o r in the carpus, the occurrence at the age of 80 years, the higher incidence of multicentricity, as
well as the recurrence rate following intralesional excision have been considered.
References 1. Averitl RM, Smith RJ, Campbell CJ (1980) Giant cell tumors of the bones of the hand. J Hand Surg 5:39 2. Campanacci M, Baldini N, Boriani S, Sudanese A (1987) Giant cell tumor of bone. J Bone Joint Surg [Am] 69:106 3. Carrasco CH, Murray JA (1989) Giant cell tumors. Orthop Clin North Am 20 (63) : 395 4. FitzPatrick DJ, Bullough PG (1977) Giant cell tumor of the lunate bone: a case report. J Hand Surg 2:269 5. Jaffe HL, Lichtenstein L, Portis RB (1940) Giant cell tumor of bone. Its pathologic appearance, grading, supposed ovarians and treatment. Arch Pathol 30 : 993 6. McDonald D J, Sim FH, McLeod RA, Dahlin DC (1986) Giant cell tumor of bone. J Bone Joint Surg [Am] 68:235 7. Moser RP, Kransdorf MJ, Gilkey FW, Manaster BJ (1990) From the archives of the AFIP giant cell tumor of the upper extremity. Radiographics 10:83
